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What is moyamoya?

Moyamoya is a disorder of the arteries of the brain where there is progressive narrowing which can lead to stroke, seizures and loss of neurologic function.  The compensatory vessels that develop are very small and fragile. Their appearance on cerebral angiography has the appearance of a "puff of smoke" hence the term "moyamoya" which is the Japanese translation. Most cases have no cause. The remainder can be due to conditions such as sickle cell disease, Down's syndrome, and neurofibromatosis type I. Females are more than twice as likely as males to have moyamoya.


How is moyamoya diagnosed?

Once a child exhibits symptoms suspicious for moyamoya, imaging is usually obtained to look at the brain blood vessels. MRI and magnetic resonance angiography (MRA) are often used. Cerebral angiography is the definitive test to establish the diagnosis and aid in management/treatment planning.

How is moyamoya treated?

In general, patients with moyamoya require treatment to provide more blood flow to the brain. Medical treatment often includes aspirin and aggressive hydration. Surgery may also help to increase brain blood flow and there are a variety of procedures that can be done to do so. A thorough consultation and medical evaluation is essential to properly diagnose moyamoya and establish a comprehensive treatment plan.

Pial Synangiosis

At Dartmouth-Hitchcock Medical Center, we are experts at diagnosing and treating adults and children with moyamoya. We have extensive experience in the imaging, evaluation and procedures necessary to provide the best possible outcome Through our multidisciplinary center, we provide resources for comprehensive treatment, long-term follow-up and support for patients and families.

For more information, please contact us.

Page reviewed on: Sep 02, 2014

Page reviewed by: Robert J. Singer, MD

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